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Year : 2022  |  Volume : 23  |  Issue : 1  |  Page : 74-76

Morpheaform leukemia cutis in a case of b-cell acute lymphoblastic leukemia - A rare presentation

1 Department of Dermatology, IMS and SUM Hospital, Bhubaneswar, Odisha, India
2 Department of Hematology, IMS and SUM Hospital, Bhubaneswar, Odisha, India

Date of Submission28-Oct-2020
Date of Decision26-Nov-2020
Date of Acceptance21-Oct-2021
Date of Web Publication31-Dec-2021

Correspondence Address:
Arunima Ray
Department of Dermatology, IMS and SUM Hospital, K-8, Kalinga Nagar, Bhubaneswar - 751 003, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.ijpd_162_20

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Leukemia cutis (LC) is the cutaneous infiltration of neoplastic cells rarely seen with acute lymphocytic leukemia. We report the case of a young female presenting with a morpheaform plaque on the face, subsequently diagnosed with B-cell acute lymphoblastic leukemia (ALL) and LC, on histopathology and immunohistochemistry. Among the limited cases of LC reported with B-cell ALL, such an atypical presentation has previously never been reported.

Keywords: B-cell acute lymphoblastic leukemia, leukemia cutis, morpheaform

How to cite this article:
Mohapatra L, Ray A, Patra PC, Samal P, Samal A, Kar BR. Morpheaform leukemia cutis in a case of b-cell acute lymphoblastic leukemia - A rare presentation. Indian J Paediatr Dermatol 2022;23:74-6

How to cite this URL:
Mohapatra L, Ray A, Patra PC, Samal P, Samal A, Kar BR. Morpheaform leukemia cutis in a case of b-cell acute lymphoblastic leukemia - A rare presentation. Indian J Paediatr Dermatol [serial online] 2022 [cited 2022 Jan 16];23:74-6. Available from: https://www.ijpd.in/text.asp?2022/23/1/74/334669

  Introduction Top

Leukemia cutis (LC) is the cutaneous infiltration of neoplastic cells usually manifesting as papules, nodules, and occasionally as plaques. It is commonly associated with underlying myelogenous leukemia, rarely seen with acute lymphocytic leukemia.[1] Hereby, we report an interesting case of an adolescent female with a solitary indurated facial plaque, subsequently diagnosed as B-cell acute lymphoblastic leukemia (ALL).

  Case Report Top

A 13-year-old girl presented with a single, well-defined, and indurated plaque of size 6 × 4 sq. cm on the left preauricular area for the last 6 months. On palpation, it was woody, hard in consistency, and mildly tender with local rise of temperature. The overlying skin looked shiny, depressed, adherent, and hyperpigmented [Figure 1]. Fingers could be insinuated beneath the plaque margin. There was a single-firm palpable left supraclavicular lymph node (1 × 1 sq. cm). Pallor was present.
Figure 1: Single morpheaform plaque on left preauricular area

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Examination of the chest and cardiovascular system revealed no major abnormality. On examination, the abdomen was soft and nontender without organomegaly.

Complete blood count showed pancytopenia with hemoglobin of 9.8 (normal 12–15 g/dl), total leukocyte count of 1.81 × 103 (normal 1–4 × 103/μl), and total platelet count of 0.82 × 106/μl (normal 1.5–4.5 × 106/μl). Peripheral smear showed hyperchromasia, anisocytosis, microcytosis, and 18% blast cells (medium size, with high nucleocytoplasmic ratio, round to slightly irregular nuclei, open chromatin, 0–1 nucleoli, and scanty cytoplasm). Other routine biochemical tests for renal function, liver function, and serum electrolytes were within normal limits. Ultrasonography of the abdomen–pelvis and chest X-ray revealed no abnormalities.

Clinically, the following differential diagnoses (LC, zygomycosis, panniculitis, and pansclerotic morphea) were considered. After taking consent from parents and patient, a punch biopsy was done and histopathology showed pandermal infiltration of small round cells. The infiltrate was perivascular and periadnexal in distribution with adnexal destruction. These small cells had hyperchromatic nuclei, inconspicuous nucleoli, and scant cytoplasm. The epidermis and subcutaneous fat were uninvolved ruling out pansclerotic morphea and lupus panniculitis. A negative periodic acid–Schiff stain excluded zygomycosis [Figure 2]a and [Figure 2]b. The findings were suggestive of small cell lymphoma. Immunohistochemistry was positive for (leukocyte common antigen: CD45), Vimentin, terminal deoxynucleotidyl transferase, CD99, and CD20, which confirmed B-cell origin of the leukemic infiltration [Figure 3]. In view of the above-described findings, she was referred to hemato-oncology department for further management. Bone marrow aspiration showed hypercellular marrow with 80% blast cells and negative myeloperoxidase stain. Marrow blast cells were positive for CD10, CD19, CD20, CD58, CD66c, CD38, NuTdt, human leukocyte antigen-DR, and CD13. These findings confirmed B-cell ALL. Conglomerating our positive results, a diagnosis of B-cell ALL with LC was made. She was started on chemotherapy by the hemato-oncologist and is under follow-up.
Figure 2: (a) Uninvolved epidermis with pandermal infiltration of small round cells (H and E stain, ×10). (b) Small cells with hyperchromatic nuclei and scant cytoplasm, relatively concentrated in perivascular and periadnexal area. (H and E stain, ×40)

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Figure 3: Immunohistochemical study; positive staining with (a) CD20, (b) CD45, (c) Terminal deoxynucleotidyl transferase, and (d) CD99. (Immunohistochemical ×40)

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  Discussion Top

LC is the infiltration of neoplastic cells and their precursors into the skin and subcutaneous tissue. Other nonspecific cutaneous manifestations of underlying leukemia are leukemids, which include purpura, ecchymoses, and paraneoplastic manifestations such as neutrophilic dermatoses and skin signs of antineoplastic drug therapy.[2],[3] As stated by Huang et al., LC is seen with 10%–15% of acute myeloid leukemia (AML), 4%–20% of chronic myeloid leukemia, and 1%–3% of ALL. In cases of ALL, majority are when the lymphocytic leukemia is of T-cell origin.[4]

The underlying mechanisms of leukemic cell infiltration are unclear. It could be explained by the selective homing of memory T cells aided by chemokines, integrins, and adhesion molecules.[5] There is cutaneous lymphocyte antigen-mediated dermal transmigration of T-cells in myelogenous leukemia, which explains the relatively higher chances of an associated LC.[6] Furthermore, abnormalities in chromosome 8 are frequently seen in AML, and they significantly predispose to LC.[7]

Clinically, LC most commonly presents as papules and nodules followed by plaques as seen in our patient. The commonly affected sites include the trunk, extremities, and face. A similar case of infiltrative plaques on the bilateral face was reported by Cotter et al., in an elderly male.[1] To the best of our knowledge, only 15 such cases have been reported so far, summarized in [Table 1].
Table 1: Reports of leukemia cutis in Acute Lymphoblastic Leukemia

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Typical histopathological features are the presence of small oval atypical cells with prominent nucleoli and scanty cytoplasm, in a perivascular and periadnexal distribution, as seen in our patient.[10]

LC foretells a poor prognosis signifying extramedullary involvement of the tumor, with poor response to chemotherapeutic agents, and a higher chance of relapse, because, the skin becomes a sanctuary and reservoir for leukemic cells and serves as a source of prolonged subclinical involvement of the marrow and other systems, even after initiation of chemotherapeutic treatment.

  Conclusion Top

Our case draws attention to a clinically confounding presentation in a young girl that can closely mimic infectious conditions (zygomycosis) and connective tissue disorders (morpheaform). The early diagnosis of LC has therapeutic and prognostic significance.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Cotter DG, Hinds BR, Orme C. A precocious presentation of B-cell acute lymphoblastic leukemia as infiltrated plaques on the face. Cureus 2019;11:e4021.  Back to cited text no. 1
Jiang X, Wang W, Zhang M. Leukemia cutis: An unusual presentation of acute lymphoblastic leukemia in a child. Indian J Dermatol 2015;60:636.  Back to cited text no. 2
[PUBMED]  [Full text]  
Haidari W, Strowd LC. Clinical characterization of leukemia cutis presentation. Cutis 2019;104:326-30.  Back to cited text no. 3
Huang YY, Liu M, Ruth JS, Potenziani S, Hsu S. Precursor-B-cell-ALL leukemia cutis resembling lipomas: An atypical presentation of a rare entity and a review of the literature. Dermatol Online J 2017;23(3). [doi: 23:13030/qt6bs2d542].  Back to cited text no. 4
Chang YW, Lee CH, Tseng HC. Leukemia cutis in a medical center in southern Taiwan: A retrospective study of 42 patients. J Formos Med Assoc 2021;120:226-33.  Back to cited text no. 5
Cho-Vega JH, Medeiros LJ, Prieto VG, Vega F. Leukemia cutis. Am J Clin Pathol 2008;129:130-42.  Back to cited text no. 6
Cronin DM, George TI, Sundram UN. An updated approach to the diagnosis of myeloid leukemia cutis. Am J Clin Pathol 2009;132:101-10.  Back to cited text no. 7
Thomas RM, Harrell JE, Rudnick E, Auerbach J, DeBenedetto A, Torres A, et al. Leukemia cutis mimicking tumid lupus as the presenting sign in a case of mixed T/B-cell acute lymphoblastic leukemia. JAAD Case Rep 2020;6:598-602.  Back to cited text no. 8
Fichel F, Eschard C, Zachar D, Munzer M, Bernard P, Grange F. Kaposiform haemangioendothelioma associated with B-cell acute lymphoblastic leukemia. Ann Dermatol Venereol 2013;140:209-14.  Back to cited text no. 9
Rao AG, Danturty I. Leukemia cutis. Indian J Dermatol 2012;57:504.  Back to cited text no. 10
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  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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